The adult with congenital heart disease

Revista Mexicana de
O
Cardiología
R
Vol. 26 No. 4
October-December 2015
The adult with congenital heart disease
El adulto con cardiopatía congénita
Lucelli Yáñez-Gutiérrez,* Diana López-Gallegos,* Carmen Emma Cerrud-Sánchez,*
Horacio Márquez-González,* Marlenne Berenice García-Pacheco,*
Moisés Jiménez-Santos,** Jaime Alfonso Santiago-Hernández,***
Homero Alberto Ramírez-Reyes,*** Carlos Riera-Kinkel****
Key words:
Congenital heart
disease, adults,
complications of
treatment, follow-up
of congenital heart
diseases.
Palabras clave:
Cardiopatía
congénita, adultos,
complicaciones
del tratamiento,
seguimiento de
las cardiopatías
congénitas.
* Servicio de
Cardiopatías
Congénitas.
** Servicio de
Tomografía.
*** Servicio de
Hemodinamia.
**** División de
Cirugía Cardiotorácica.
UMAE Hospital de
Cardiología Centro
Médico Nacional Siglo
XXI.
ABSTRACT
RESUMEN
Background: The study show the most important demographic and clinical data of a cohort composed by adults
treated in a congenital heart clinic. Material and methods: In a retrospective, observational and longitudinal
study, data from patients with congenital heart disease,
older than 18 years, seen between May 2010 and May
2013, were analyzed. Results: The cohort comprised 409
patients, 280 of them female (69%), whose mean age at the
time of the first evaluation was 36.7 ± 14.2 years (range,
18 to 75 years). Thirty-four of them (8%) had cyanotic
congenital heart disease (14 cases of tetralogy of Fallot,
the rest with a univentricular heart and Eisenmenger). The
mean age when heart disease was diagnosed was 33.6 ±
15.9 years (range, from birth to 75 years). One hundred
five patients (38%) were aware of having heart disease
but did not have regular follow-up. The reason for referral
was heart murmur in 143 subjects (35%); in 143 (35%) for
deterioration of functional class due to progressive dyspnea; supraventricular arrhythmias in 24 (6%); chest pain
in 16 (4%), in 12 arterial hypertension (3%) and in 8 (2%)
history of cerebral vascular event. On regard of the types
of congenital heart disease, 71% were intracardiac defects
(mainly interatrial and interventricular septal defects),
10% left and right obstructive lesions, as well as aortic
coarctation, 7% mixed valve lesions, 2% cardiomyopathy,
and the rest with diseases like anomalous origin of the left
coronary artery, double discordance and univentricular
heart. In 188 patients (46%) surgical treatment was opted,
meanwhile in other 138 (34%) interventional percutaneous
treatments were chosen, and in 83 (20%) medical treatment was selected. Fifteen patients suffered complications
related to treatment, 50% of them infections, arrhythmias
or heart failure. Mortality occurred in six cases, all from
the surgical group. The cause of death was refractory heart
failure and cardiogenic shock in the postoperative phase.
Pulmonary pressure descended from 48.3 ± 20.4 mmHg
before the therapeutic procedure to 36.3 ± 16.2 mmHg
Antecedentes: Este estudio muestra los datos clínicos y
demográficos más importantes de una cohorte compuesta
de pacientes tratados en una clínica de cardiopatías congénitas. Material y métodos: Fueron analizados los datos
de pacientes con cardiopatías congénitas, mayores de 18
años, vistos entre los meses de mayo del 2010 al 2013,
en un estudio retrospectivo, observacional y longitudinal.
Resultados: La cohorte comprendió 409 pacientes, 280 de
ellos del género femenino (69%), con promedio de edad
de 36.7 ± 14.2 años (rango de 18 a 75 años). Treinta y
cuatro de ellos (8%) tenían cardiopatía congénita cianótica
(14 con tetralogía de Fallot, el resto con corazón univentricular o Eisenmenger). El promedio de edad en la que
fue diagnosticada la cardiopatía fue de 33.6 ± 15.9 años
(rango, desde el nacimiento a los 75 años). Ciento cinco
pacientes (38%) conocían que tenían cardiopatía, pero no
tenían un seguimiento regular. La razón de la referencia fue
en 143 individuos (35%) un soplo cardiaco, en otros 143
(35%) por deterioro de la clase funcional debido a disnea
progresiva; arritmias supraventriculares en 24 (6%); dolor
torácico en 16 (4%), hipertensión arterial en 12 (3%) y en
8 (2%) historia de evento vascular cerebral. En cuanto al
tipo de cardiopatías congénitas en 71% de los casos fueron
defectos intracardiacos (principalmente, comunicaciones
interauriculares e interventriculares), 10% de lesiones
obstructivas derechas e izquierda, así como coartación de
la aorta; 7% de lesiones valvulares combinadas; 2% de
cardiomiopatías y el resto que comprendió enfermedades
como nacimiento anómalo de la coronaria izquierda, doble
discordancia y corazón univentricular. En 188 pacientes
(46%) los pacientes fueron tratados quirúrgicamente, en
tanto que en 138 (34%) se decidió por la intervención
percutánea y en 83 (20%) más, por el tratamiento médico.
Quince pacientes tuvieron complicaciones relacionadas
con el tratamiento, 50% de ellas infecciones, arritmias o
insuficiencia cardiaca. Seis pacientes del grupo quirúrgico
murieron, debido a insuficiencia cardiaca refractaria o
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Yáñez-Gutiérrez L. The adult with congenital heart disease
after it (p < 0.001). Conclusions: Adults with congenital
heart disease form a complex study group that in addition
to the underlying disease, have a combination of other diseases and/or cardiovascular risk factors as well as several
complications related to previous therapeutic procedures.
INTRODUCTION
Pediatric cardiology is a relatively young and
booming branch of cardiovascular medicine
with exponential growth in recent years. Due
to advances in surgical techniques, improved
devices used in interventional cardiology and
better postoperative care of patients, survival
rates in patients with congenital heart disease
(CHD) have increasead.1 It is estimated that a
great proportion of children with CHD reach
adulthood, to the point that, for the first time,
the number of adults with congenital cardiopathies exceeds the amount of children with the
same pathology.2,3
Only in the United States there are about
one million adult patients with CHD, with an
annual growth rate of 5%. In that country, every
year 50,000 more of these patients will require
attention. In our country, we can estimate
300,000 cases of adults with CHD, with an
annual increase of 15%.4
The largest group of adult patients with
CHD is composed by those patients who underwent some kind of therapeutic intervention
during childhood. This is the reason why we
are now detecting more late complications of
these interventions. Reasons for referral in this
group of patients are mainly heart failure, endocarditis, diverse arrhythmias and pregnancy.5
The scope of this study is to assess and
analyze some clinical data in a cohort of adult
patients with CHD followed-up in the outpatient clinic of our service.
choque cardiogénico en el postoperatorio. La presión de
la arteria antes y después del procedimiento terapéutico
bajó de 48.3 ± 20.4 a 36.3 ± 16.2 mmHg (p < 0.001).
Conclusiones: Los adultos con cardiopatía congénita
forman un grupo de estudio complejo, pues en ellos se
agregan a la enfermedad de base otras enfermedades y
factores de riesgo cardiovascular, más las complicaciones
relacionadas con los procedimientos terapéuticos previos.
vice of congenital heart disease of our service, in
the period from May 2010 to May 2013 were
analyzed. In addition to demographic data, we
took into consideration the type of CHD, the
reason of referral to our service and the kind
of therapeutic strategy employed: surgical,
interventional or medical.
Descriptive basic statistic methods were
employed to measure central tendency and
dispersion, according to distribution and inferential statistics.
RESULTS
The clinical data of 409 patients were analyzed,
280 of them were female (69%). The age at
the time of evaluation was 36.7 ± 14.2 years
(range 18-75 years).
At the time of evaluation, there were only
34 cases of cyanotic CHD (8%); of these, 14
patients had tetralogy of Fallot, 10 univentricular heart and 10 Eisenmenger.
The age at the time when the diagnosis of
CHD was established was 33.6 ± 15.9 years
on average (ranging from birth to 75 years).
Of all patients, 38% patients knew they had
heart disease and failed to undergo follow up,
mostly because they lost their pertaining to
social security.
The main reasons for referral were: heart
murmur, deterioration of the New York Heart
Association (NYHA) functional class due to dyspnea, arrhythmia, chest pain, systemic arterial
hypertension, and presence of cerebral vascular
event (Figure 1).
Figure 2 shows the main diagnoses in order
of frequency: intracardiac septal defects (290),
left side obstructive lesions (41), valve lesions
(29), cardiomyopathies (8) and mixed lesions
(41).
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MATERIAL AND METHODS
The present study is retrospective, observational
and longitudinal. Data from clinical records of
patients older than 18 years, treated in the ser-
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Yáñez-Gutiérrez L. The adult with congenital heart disease
Figure 3 provides a breakdown of the most
common diagnoses seen during the observation interval.
Figure 4 shows the type of therapeutic options employed during the observation time.
Surgical treatment included: closure of atrial
or ventricular septal defects in two cases associated with myocardial revascularization
and implantation of mechanical prosthesis.
Mechanical prosthesis placement was either for
200
180
172
160
143
140
120
100
80
60
40
24
20
0
Heart
murmur
Dyspnea
16
Arrhythmia
Chest
pain
12
8
Systemic Cerebral
vascular
arterial
hypertension event
(CVE)
(SAH)
Figure 1. Reason reference to the service of congenital heart disease.
350
300
290
250
DISCUSSION AND CONCLUSIONS
200
150
100
41
50
0
the first time or involved valved tube or valve
replacements. Reoperation included interventions to correct residual defects or total reparation and was mostly for tetralogy of Fallot or,
double outlet right ventricle including a small
group of adult patients who undergo complete
Fontan surgery.
The interventional treatment mostly encompassed closing atrial septal defects and patent
ductus arteriosus but also included aortoplasty
patients with angioplasty and stenting of pulmonary peripheral branches, as well as a small
but significant number of cases in which embolization of collateral arteries was performed
as a preparation for completing univentricular
heart surgery. And finally, in the medical treatment group were included patients with heart
failure, pulmonary hypertension or cardiac
arrhythmias, whatever this type of treatment
was the only alternative or concomitantly with
either of the other two options.
Special mention should be made of the
group of patients with short circuits, essentially
interatrial septal defect with pulmonary artery
systolic pressure (PASP > 40 mmHg) as they
all underwent cardiac catheterization study
with vascular reactivity tests with nitric oxide.
Those who showed a decrease in pulmonary
artery pressure were considered candidates for
a surgical technique with valved patch pulmonary pressure showed a statistically significant
decrease of 48.3 ± 20.4 versus 36.3 ± 16.2
(p < 0.001).
In the surgical group occurred six deaths for
cardiogenic shock, and four cases for infection,
arrhythmias and heart failure.
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41
29
8
Intracardiac
septal
defects
Six to eight children are born with heart problems for each 1,000 live births. Half of the
children with CHD require urgent attention in
the first year of life, while the majority of the
rest will require, at some time, medical specialized attention or therapeutic intervention. As it
has been mentioned previously, the fact that 8
out of 10 reach patients with CHD will reach
adulthood, thanks to advances in diagnostic
and therapeutic techniques,1 explains that for
the first time the amount of adults with CHD
exceeds the number of pediatric patients with
Left side
obstructive
lesions
Valve
lesions
Cardiomyopathies
Figure 2. Most frequent diagnoses in adult congenital heart service.
Rev Mex Cardiol 2015; 26 (4): 158-162
Mixed
lesions
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Yáñez-Gutiérrez L. The adult with congenital heart disease
Diagnoses
Most common diagnoses
Figure 3.
Aneurysm
7
Double aortic lesion
8
Tetralogy of Fallot
15
Intraventricular communication
15
Permeable foramen oral (PFO)
17
Ebstein
17
Ductus arteriosus persistence
24
Aortic coarctation
Most common diagnoses seen during the
observation interval.
29
Other
65
212
Atrial septal defect (ASD)
0
50
Medical treatment
20%
Surgical treatment
46%
Interventional
treatment
34%
Figure 4.
Treatment options in
the adult congenital
heart disease group.
these diseases. In consequence an increased
number of adult patients with CHD now are
seen and studied in cardiology departments,
making it necessary to know better the physiopathology and potential complications of these
heart problems, as well as the therapeutic options (surgical or interventional) they require.2
A significant number of patients were not
aware of their heart disease, while about a third
of them indeed had the information that they
had received specialized attention, at some
point in their lives. In spite of this fact, many
of them had not been under regular follow-up,
either because they consider the heart problem
essentially benign or for administrative reasons,
mainly the loss of their right to social security
medical care from the moment they reach
adulthood. In the group of patients with recent
diagnosis of CHD, the most numerous are those
100
150
Number of cases
200
250
with atrial septal defects or patent ductus arteriosus, because, mostly of all, atrial septal defects
(and less commonly patent ductus arteriosus)
are well tolerated during childhood and adolesdeveloping
symptoms
around the 4th or
Estecence,
documento
es elaborado
por Medigraphic
5th decade of life (generally the clinical manifestations were the deterioration of functional
class due to dyspnea and cardiac arrhythmias,
generally of supraventricular origin).6,7
The group of patients undergoing intervention included those with left obstructions either
valve or aortic coarctation. Also, tetralogy of
Fallot appears as the first complex heart disease treated in adulthood after being operated
during childhood. Furthermore, a very small
number of patients with univentricular heart
syndrome were included.8,9
Similar to what happens in the pediatric
age, in adults a very common cause of referral
is the detection of a cardiac murmur during a
routine examination, but in the latter age this
clinical finding is more frequently associated
to a decline in functional class due to dyspnea
on moderate efforts, as well as the apparition
of cardiac arrhythmias.1
Unlike what happens in childhood, in adults
with CHD surgery is the first choice for many
diseases. But unfortunately, there is a large
group of patients in whom surgical procedures
are not indicated because the coexistence of
heart failure or pulmonary arterial hypertension
secondary to voluminous systemic-pulmonary
shunts caused by large defects. When pulmonary vessels are severely damaged and are
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Yáñez-Gutiérrez L. The adult with congenital heart disease
unresponsive to pulmonary vasodilators, in general, curative procedures have to be ruled out.
And finally, in a very small number of patients,
especially those with complex heart disease,
a combination of surgical, interventional and
medical procedures could be necessary.7,8
According to the above mentioned, it is
necessary to delve in the natural history of
these diseases, in the possible therapeutic
approaches and their inherent complications,
because the initial anatomic and physiological
complexities of some CHD are entangled in the
adult to other acquired heart diseases, to the
effects of pregnancy and to the late complications of the therapeutic procedures. In order to
provide the better care to these rather complex
patients a multidisciplinary team is mandatory
formed by pediatric and adult cardiologists,
echocardiographers, interventional, and electrophysiology cardiologists, as well as cardiac
surgeons, intensive care specialists, psychologists, geneticist, etc.10-12
The challenge of the future is to raise consciousness in everyone for earlier diagnosis and
timely treatment. Also is needed to inculcate
in the parents of children with CHD the strict
compliance of the following-up strategies,
beginning with regular and periodical medical
visits. These are diseases of lifetime duration,
needing constant supervision and guidance.
Finally, is highly desirable the creation of multidisciplinary clinics able to bring high quality
care to these patients, as well as constitute
themselves in centers for promoting education
and research, encouraging effective communications between all elements of the health
team.2,13
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Correspondence to:
Lucelli Yáñez Gutiérrez MD
Cuauhtémoc Núm. 330, Col. Doctores,
Del. Cuauhtémoc, 06760, Mexico City
Phone: (55) 56-27-69-00, Local: 22203
E-mail: lucelli.yanez@imss.gob.mx
www.medigraphic.org.mx
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